Advancements in the Treatment and Management of Voiding Dysfunction in Individuals with Spina Bifida.

Putting Adolescent Health at the Heart of Pediatrics

This commentary is on the original article by Oakeshott et al. on pages 634–638 of this issue.

SPINA BIFIDA AS AN INDEPENDENT RISK FACTOR FOR SENSITIZATION TO LATEX

Development and Validation of the Fecal Incontinence and Constipation Quality of Life Measure in Children With Spina Bifida

Splitting birth defects into dysmorphologically homogeneous groups might improve the ability to detect a genetic risk factor or teratogenic exposure. With regard to spina bifida, recent studies suggest that etiologic heterogeneity exists within the group of spina bifida, although exogenous risk factors have been sparsely evaluated for subgroups. In the present study, 210 spina bifida patients were classified into relatively homogeneous groups, based on retrospective information on appearance and functional aspects of the lesion abstracted from medical records of the patients. We compared high with low spina bifida, and open with closed spina bifida, and investigated whether risk factors for spina bifida such as maternal age, antiepileptic drug use, parental occupation, and genetic factors were specifically associated with these homogeneous subclasses. For these comparisons, a referent group of 671 children was used. Although classifying spina bifida into homogeneous subclasses presented some difficulties and numbers were small, this study provides some evidence for different risk profiles for subclasses of spina bifida. The sex ratio, the proportion of miscarriages of siblings, and maternal age did not differ among the different subclasses of spina bifida. However, children with a positive family history of neural tube defects (NTDs) had a higher risk of high spina bifida [odds ratio (OR) = 6.3, 95% confidence interval (CI): 1.7-19.2] than of low spina bifida (OR = 2.1, 95% CI: 1.0-4.2). Siblings with NTDs were more common in cases with high spina bifida and cases with open spina bifida. A strongly increased risk of high spina bifida was found for male welders (OR = 12.1, 95% CI: 1.5-64.2), whereas the risk of low spina bifida was much lower (OR = 1.6, 95% CI: 0.2-7.9). For mothers with agricultural occupations, a strongly increased risk was observed for open spina bifida (OR = 14.3, 95% CI: 2.9-77.7), whereas none of 107 cases with closed spina bifida had a mother with an occupation in agriculture. Due to small numbers, the results must be interpreted with caution.

Individuals living with cerebral palsy or spina bifida are at heightened risk for a number of chronic health conditions, such as secondary comorbidities, that may develop or be influenced by the disability, the presence of impairment, and/or the process of aging. However, very little is known about the prevalence and/or risk of developing secondary comorbidities among individuals living with cerebral palsy or spina bifida throughout adulthood. The objective of this study was to compare the prevalence of psychological, cardiometabolic, and musculoskeletal morbidity and multimorbidity among adults with and without cerebral palsy or spina bifida. Privately insured beneficiaries were included if they had an International Classification of Diseases, Ninth Revision, Clinical Modification diagnostic code for cerebral palsy or spina bifida (n = 29,841). Adults without cerebral palsy or spina bifida were also included (n = 5,384,849). Prevalence estimates of common psychological, cardiometabolic, and musculoskeletal morbidity and multimorbidity (≥2 conditions) were compared. Adults living with cerebral palsy or spina bifida had a higher prevalence of all psychological disorders and psychological multimorbidity (14.6% vs. 5.4%), all cardiometabolic disorders and cardiometabolic multimorbidity (22.4% vs. 15.0%), and all musculoskeletal disorders and musculoskeletal multimorbidity (12.2% vs. 5.4%), as compared with adults without cerebral palsy or spina bifida, and differences were to a clinically meaningful extent. Adults with cerebral palsy or spina bifida have a significantly higher prevalence of common psychological, cardiometabolic, and musculoskeletal morbidity and multimorbidity, as compared with adults without cerebral palsy or spina bifida. Efforts are needed to facilitate the development of improved clinical screening algorithms and early interventions to reduce risk of disease onset/progression in these higher risk populations. Complete the self-assessment activity and evaluation online at http://www.physiatry.org/JournalCME. Upon completion of this article, the reader should be able to: (1) List the main categories of morbidity that present with higher risk in adults with cerebral palsy and spina bifida; (2) Discuss the potential impact of multimorbidity on 'early aging' in adults living with cerebral palsy and spina bifida; and (3) Describe challenges that adults with cerebral palsy and spina bifida have in obtaining appropriate health care to address prevention and treatment of multimorbidity. Advanced. The Association of Academic Physiatrists is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.The Association of Academic Physiatrists designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Pregnancy among mothers with spina bifida

Establishing the first community-centered Spina Bifida research agenda

Characteristics and Survival of Patients with End Stage Renal Disease and Spina Bifida in the United States Renal Data System

ObjectivesTo characterize the estimated prevalence and treatment patterns of erectile dysfunction and hypogonadism in men with spina bifida through a large claims database.MethodsThis retrospective claims study used the MarketScan® databases from 2008 to 2017 to compare prevalence estimates for erectile dysfunction and hypogonadism in men with spina bifida to those in men without spina bifida and to describe treatment patterns in this cohort.ResultsThe estimated prevalence of erectile dysfunction and hypogonadism in men with spina bifida was 7.83% and 7.71%, respectively. Men with spina bifida exhibit high rates of smoking and metabolic comorbidities but are diagnosed less frequently with erectile dysfunction when controlling for age and metabolic comorbidities than men without spina bifida. Most men with spina bifida and erectile dysfunction (66.6%) or hypogonadism (77.4%) do not receive treatment. However, a diagnosis of spina bifida did not appear to affect the likelihood of treatment for either condition on multivariate analysis.ConclusionsMen with spina bifida are known to be at high risk for erectile dysfunction but may be diagnosed or treated less frequently than age and disease-matched men without spina bifida. Hypogonadism is diagnosed more frequently in men with spina bifida, which is a new finding that warrants further investigation. Most men with SB and either ED or HG do not receive treatment. The results suggest there is potential for improving care delivery for sexual health issues in men with spina bifida.

Assessment of health literacy in adolescents with spina bifida and their caregivers: a multi-institutional study

The 53rd Annual Meeting of the Society for Research into Hydrocephalus and Spina Bifida was held at Queen's University in Belfast, Northern Ireland at the invitation of the local organizing committee; Stephen Brown, Nan Hill, John McCann, Alan Bailie, David Marshall, Tabib Dabir, Emma Kelly and Marie McGonnell. Proceedings began as usual on Wednesday afternoon with the Annual General Meeting of Members. This was followed by a reception for all delegates and guests where we were welcomed to Belfast by the Lord Mayor, Councillor Naomi Long. The scientific programme was opened by a welcome from Dr. Michael McBride, Chief Medical Officer for Northern Ireland, and included sessions on 'Spina Bifida: Incidence, Survival and Cognitive Outcome', 'Hydrocephalus', 'Animal Studies', 'Hydrocephalus Shunts', 'Long Term Management of Spina Bifida Patients', 'Urology and Bowel Management' and 'Spina Bifida'. We also had two Invited Lectures, firstly from Neil Buxton (Liverpool, UK), on 'Pain Management in Spina Bifida: a Neurosurgical Perspective' and from Professor Sivert Lindstrom, (Linkoping, Sweden) on 'The Bladder Cooling Test in Spina Bifida'. Retiring President, Professor Ray Fitzgerald (Dublin, Ireland) gave his valedictory lecture entitled 'Here and There with Hydrocephalus', describing changes in treatment he has witnessed through his long and distinguished career. Friday morning's programme included a parallel break-out session on 'Long-term Management of Children with Spina Bifida', aimed at Nurses and Health Care Professionals. The social programme continued on Thursday afternoon with a visit to the Thompson Graving Dock. This dry dock is where RMS Titanic underwent a final hull inspection in February and March 1912 before final sea trials and sailing to Southampton before sailing on her maiden voyage on Wednesday 10th April. She hit an iceberg on the night of Sunday 14th April and sank with the loss of over 1,500 lives. The afternoon continued with a visit to Stormont, the home of the Northern Ireland Assembly at the invitation of Michael McGimpsey, Minister of Health. We were able to visit both assembly chambers and were then entertained in the Great Hall by Belfast's Open Arts Community Choir, a nationally acclaimed choir featuring people with disabilities and those without, and traditional Irish music from The O'Malley Experience. The final event of the social programme was a gala dinner in the magnificent Great Hall of Queen's University.

Colon Enemas for Fecal Incontinence in Patients with Spina Bifida

Self-perceived health among children with spina bifida in the West Bank: a cross-sectional study

Introduction Spina Bifida is a congenital neurological defect associated with genitourinary anomalies, requiring comprehensive urological care from pediatrics to adulthood. For men with spina bifida, a significant focus is on urinary tract surveillance to maintain continence, preserve kidney and bladder health, and optimize fertility and sexual function. While men with SB have an increasing interest in exploring their sexuality and sexual health, these concerns may be under-discussed and undertreated depending on their burden of illness. ED is associated with diminished body image and low self-confidence resulting in poor sexual health. Additionally, it creates barriers to maintaining intimate relationships. Current data tackling the prevalence of erectile dysfunction demonstrates variations in value, yet low quantifications of treatment for SB patients. Prior studies have shown a positive response to phosphodiesterase-5 (PDE5) inhibitor therapy (80%). Concurrently, the impact of hypogonadism on the SB is unknown. Hypogonadism is associated with increased insulin resistance in young males, hyperlipidemia, and other adverse vascular effects. These risk factors play a role in the vascular etiology of erectile dysfunction and can be a burden on sexual health. With a scarcity of men’s sexual health data focused on the adult SB male population, there is a need to illustrate the prevalence of ED and HG in men with SB while investigating treatment utilization. We hypothesize that men with SB will experience a higher rate of diagnosis of ED and HG compared to the general adult male population, and lower treatment utilization rates. Objective To calculate the estimated prevalence of erectile dysfunction and hypogonadism in adult men with spina bifida Methods This retrospective claims study used the MarketScan® databases from 2008 to 2017 to derive prevalence estimates for erectile dysfunction and hypogonadism in men with spina bifida, to compare these estimates to those in men without spina bifida, and to describe treatment patterns in this cohort. All men in aged 18 years and older with a diagnosis of SB were included for analysis. Patients with congenital hormonal disorders were excluded. To analyze treatment utilization, the National Drug Codes for pharmacotherapies for ED or HG and Current Procedural Terminology (CPT) codes for penile prosthesis were used. Results The estimated prevalence of erectile dysfunction and hypogonadism in men with spina bifida was 7.83% and 7.71%, respectively. Men with spina bifida in the sample exhibited high rates of smoking and metabolic comorbidities, but were diagnosed less frequently with erectile dysfunction when controlling for age and metabolic comorbidities than men without spina bifida. Most men with spina bifida and ED (66.6%) or hypogonadism (77.4%) do not receive treatment. However, a diagnosis of spina bifida did not appear to affect the likelihood of treatment for either condition on multivariate analysis. Conclusions Based on the results, claims data suggest that HG and ED are diagnosed less frequently than expected. While there are some limitations to the population being examined in this database, improving health care delivery of sexual health concerns during the surveillance of men with spina bifida will enhance their quality of life and optimize their sexual and metabolic function. Disclosure No